Ann Hepatobiliary Pancreat Surg.  2023 Feb;27(1):107-113. 10.14701/ahbps.22-054.

Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: Three case reports and a literature review

• Sung MK
• Lee W
• Hong S
• Park Y
• Kwak BJ
• Song KB
• Lee JH
• Hwang DW
• Kim SC

• KMID: 2539521
• DOI: http://doi.org/10.14701/ahbps.22-054

Abstract

Mixed adenoneuroendocrine carcinoma is defined as a tumor with a mixture of adenocarcinoma components and neuroendocrine neoplasm components. Each of these two components of mixed adenoneuroendocrine carcinoma accounts for at least 30% of all tumors. Mixed adenoneuroendocrine carcinoma might be located in the ampulla of Vater, a very rare location compared to other organs. Thus, its treatment and prognosis plans have not been established yet. We report three cases of mixed adenoneuroendocrine carcinoma occurring in the ampulla of Vater. Each patient had a different clinical course. In general, difficulty in preoperative diagnosis, risk of early recurrence, and poor disease course were main hallmarks of mixed adenoneuroendocrine carcinoma arising from the ampulla of Vater. However, one patient in this case report survived although she did not receive adjuvant chemotherapy due to her old age. Therefore, it is important to establish a careful treatment strategy for mixed adenoneuroendocrine carcinoma arising from the ampulla of Vater.

Keyword

Adenocarcinoma; Neuroendocrine tumor; Ampulla of Vater; Immunohistochemistry

Figure

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  Fig. 1 (A) Case 1 magnetic resonance cholangiopancreatography (MRCP) findings of the neoplastic lesion showing bile duct dilatation, obstruction in the ampullary area of the stretched bile duct, a 2.3 cm long mass involving the ampulla of Vater, and lymph nodes less than 1 cm in the retropancreatic area. (B) Case 1 surgical specimen of the resected ampullary tumor.

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  Fig. 2 (A) Case 1 H&E staining showing a mixed adenoneuroendocrine tumor (x200). (B) Case 1 immunohistochemical analysis showing neuroendocrine tumor cells being positive for CD56 (x200).

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  Fig. 3 (A) Case 2 magnetic resonance cholangiopancreatography (MRCP) findings of the neoplastic lesion showing bile duct dilatation, obstruction in the ampullary area of the stretched bile duct, and a 3.0 cm long mass involving the ampulla of Vater without enlarged lymph nodes. (B) Case 2 surgical specimen of the resected ampullary tumor.

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  Fig. 4 (A) Case 3 magnetic resonance cholangiopancreatography (MRCP) findings of the neoplastic lesion showing bile duct dilatation, obstruction in the ampullary area of the stretched bile duct and main pancreatic duct dilatation, and a 1.5 cm long mass involving the ampulla of Vater without enlarged lymph nodes. (B) Case 3 surgical specimen of the resected ampullary tumor.

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